bims-hylehe Biomed News
on Hypoplastic left heart syndrome
Issue of 2019‒09‒29
seven papers selected by
Richard James
University of Pennsylvania


  1. Am J Transplant. 2019 Sep 22.
    Magnetta DA, Godown J, West S, Zinn M, Rose-Felker K, Miller S, Feingold B.
      US pediatric heart allocation policy was recently revised, deprioritizing candidates with cardiomyopathy while maintaining status 1A eligibility for congenital heart disease (CHD) candidates on "high-dose" inotropes. We compared waitlist characteristics and mortality around this change. Status 1A listings decreased (70% to 56%, P < .001) and CHD representation increased among status 1A listings (48% vs 64%, P < .001). Waitlist mortality overall (subdistribution hazard ratio [SHR] 0.96, P = .63) and among status 1A candidates (SHR 1.16, P = .14) were unchanged. CHD waitlist mortality trended better (SHR 0.82, P = .06) but was unchanged for CHD candidates listed status 1A (SHR 0.92, P = .47). Status 1A listing exceptions increased 2- to 3-fold among hypertrophic and restrictive cardiomyopathy candidates and 13.5-fold among dilated cardiomyopathy (DCM) candidates. Hypertrophic (SHR 6.25, P = .004) and restrictive (SHR 3.87, P = .03) cardiomyopathy candidates without status 1A exception had increased waitlist mortality, but those with DCM did not (SHR 1.26, P = .32). Ventricular assist device (VAD) use increased only among DCM candidates ≥1 years old (26% vs 38%, P < .001). Current allocation policy has increased CHD status 1A representation but has not improved their waitlist mortality. Excessive DCM status 1A listing exceptions and continued status 1A prioritization of children on stable VADs potentially diminish the intended benefits of policy revision.
    Keywords:  Organ Procurement and Transplantation Network (OPTN); United Network for Organ Sharing (UNOS); clinical research/practice; heart disease: congenital; heart transplantation/cardiology; organ allocation; organ procurement and allocation; patient survival; pediatrics
    DOI:  https://doi.org/10.1111/ajt.15567
  2. J Pediatr. 2019 Sep 24. pii: S0022-3476(19)30993-X. [Epub ahead of print]
    Cognata A, Kataria-Hale J, Griffiths P, Maskatia S, Rios D, O'Donnell A, Roddy DJ, Mehollin-Ray A, Hagan J, Placencia J, Hair AB.
      OBJECTIVE: To evaluate the hypothesis that feeding volumes exceeding 100 mL/kg/d and exposure to cow's milk formula preoperatively increase the risk for preoperative necrotizing enterocolitis (NEC) in infants with complex congenital heart disease.STUDY DESIGN: All infants, of any gestational age, with an isolated cardiac lesion at high risk for NEC (ductal-dependent lesions, transposition of the great arteries, truncus arteriosus, and aorto-pulmonary window) admitted to Texas Children's Hospital from 2010 to 2016 were included. NEC was defined based on the modified Bell criteria. Feeding regimen information and relevant covariates were collected. Logistic regression was used to evaluate the association of feeding regimen and other potential risk factors with NEC.
    RESULTS: In this single-center, retrospective cohort of 546 infants, 3.3% developed Bell stage I-III NEC preoperatively. An exclusive unfortified human milk diet was associated with a significantly lower risk of preoperative NEC (OR 0.17, 95% CI 0.04-0.84, P = .03) in a multivariable regression model controlling for cardiac lesion, race, feeding volume, birth weight small for gestational age, inotrope use presurgery/pre-NEC, and prematurity. Feeding volumes exceeding 100 mL/kg/d were associated with a significantly greater risk of preoperative NEC (OR 3.05, 95% CI 1.19-7.90, P = .02).
    CONCLUSIONS: The findings suggest that an unfortified exclusive human milk diet may reduce the risk of preoperative NEC in infants with complex congenital heart disease.
    Keywords:  cohort study; ductal dependent cardiac lesions
    DOI:  https://doi.org/10.1016/j.jpeds.2019.08.009
  3. Cold Spring Harb Perspect Biol. 2019 Sep 23. pii: a037234. [Epub ahead of print]
    Kalisch-Smith JI, Ved N, Sparrow DB.
      Congenital heart disease (CHD) has many forms and a wide range of causes. Clinically, it is important to understand the causes. This allows estimation of recurrence rate, guides treatment options, and may also be used to formulate public health advice to reduce the population prevalence of CHD. The recent advent of sophisticated genetic and genomic methods has led to the identification of more than 100 genes associated with CHD. However, despite these great strides, to date only one-third of CHD cases have been shown to have a simple genetic cause. This is because CHD can also be caused by oligogenic factors, environmental factors, and/or gene-environment interaction. Although solid evidence for environmental causes of CHD have been available for almost 80 years, it is only very recently that the molecular mechanisms for these risk factors have begun to be investigated. In this review, we describe the most important environmental CHD risk factors, and what is known about how they cause CHD.
    DOI:  https://doi.org/10.1101/cshperspect.a037234
  4. J Pediatr Health Care. 2019 Sep 20. pii: S0891-5245(19)30250-0. [Epub ahead of print]
    Grunberg VA, Geller PA, Patterson CA.
      INTRODUCTION: This study was conducted to examine whether the parental report of objective infant medical indices (e.g., birth weight, length of stay) can be used to identify parents at risk for psychosocial sequelae.METHODS: Parents (N = 199) cohabitating with their partner and child who was discharged from a neonatal intensive care unit (NICU) 6 months to 3 years prior to the administration of the study completed an online survey, which included parent-reported infant health, parenting stress, family burden, and family resources.
    RESULTS: A hierarchical cluster analysis identified the following three clusters of parents at risk for stress and family burden as determined by infant medical severity and access to resources: lowest risk (n = 77), moderate risk (n = 68), and highest risk (n = 8).
    DISCUSSION: This work highlights how a measure using parent-reported infant health severity, which was developed for this study, can be used to better understand family outcomes following NICU hospitalization.
    Keywords:  Neonatal intensive care unit (NICU); family burden; family resources; infant health; parental stress
    DOI:  https://doi.org/10.1016/j.pedhc.2019.07.005
  5. Eur J Cardiovasc Nurs. 2019 Sep 25. 1474515119876148
    van der Mheen M, van der Meulen MH, den Boer SL, Schreutelkamp DJ, van der Ende J, de Nijs PF, Breur JM, Tanke RB, Blom NA, Rammeloo LA, Ten Harkel AD, du Marchie Sarvaas GJ, Utens EM, Dalinghaus M.
      BACKGROUND: Dilated cardiomyopathy (DCM) in children is an important cause of severe heart failure and carries a poor prognosis. Adults with heart failure are at increased risk of anxiety and depression and such symptoms predict adverse clinical outcomes such as mortality. In children with DCM, studies examining these associations are scarce.AIMS: We studied whether in children with DCM: (1) the level of emotional and behavioral problems was increased as compared to normative data, and (2) depressive and anxiety problems were associated with the combined risk of death or cardiac transplantation.
    METHODS: To assess emotional and behavioral problems in children with DCM, parents of 68 children, aged 1.5-18 years (6.9±5.7 years), completed the Child Behavior Checklist.
    RESULTS: Compared to normative data, more young children (1.5-5 years) with DCM had somatic complaints (24.3% vs. 8.0%; p < .001), but fewer had externalizing problems (5.4% vs. 17.0%; p = .049). Overall internalizing problems did not reach significance. Compared to normative data, more older children (6-18 years) showed internalizing problems (38.7% vs. 17.0%; p = .001), including depressive (29.0% vs. 8.0%; p < .001) and anxiety problems (19.4% vs. 8.0%; p = .023), and somatic complaints (29.0% vs. 8.0%; p < .001). Anxiety and depressive problems, corrected for heart failure severity, did not predict the risk of death or cardiac transplantation.
    CONCLUSION: Children of 6 years and older showed more depressive and anxiety problems than the normative population. Moreover, in both age groups, somatic problems were common. No association with outcome could be demonstrated.
    Keywords:  Dilated cardiomyopathy; emotional and behavioral problems; heart failure; pediatrics; psychosocial support
    DOI:  https://doi.org/10.1177/1474515119876148
  6. Congenit Heart Dis. 2019 Sep 23.
    Bobillo-Perez S, Sanchez-de-Toledo J, Segura S, Girona-Alarcon M, Mele M, Sole-Ribalta A, Cañizo Vazquez D, Jordan I, Cambra FJ.
      OBJECTIVE: Three scores have been proposed to stratify the risk of mortality for each cardiac surgical procedure: The RACHS-1, the Aristotle Basic Complexity (ABC), and the STS-EACTS complexity scoring model. The aim was to compare the ability to predict mortality and morbidity of the three scores applied to a specific population.DESIGN: Retrospective, descriptive study.
    SETTING: Pediatric and neonatal intensive care units in a referral hospital.
    PATIENTS: Children under 18 years admitted to the intensive care unit after surgery.
    INTERVENTIONS: None.
    OUTCOME MEASURES: Demographic, clinical, and surgical data were assessed. Morbidity was considered as prolonged length of stay (LOS > 75 percentile), high respiratory (>72 hours of mechanical ventilation), and high hemodynamic support (inotropic support >20).
    RESULTS: One thousand and thirty-seven patients were included, in which 205 were newborns (18%). The category 2 was the most frequent in the three scores: In RACHS-1, ABC, 44.9%, and STS-EACTS, 40.8%. Newborns presented significant higher categories. Children required cardiopulmonary bypass in more occasions (P < .001) but the times of bypass and aortic cross-clamp were significantly higher in newborns (P < .001 and P = .016). Thirty-two patients died (2.8%). A quarter of patients had a prolonged LOS, 17%, a high respiratory support, and 7.1%, a high hemodynamic support. RACHS-1 (AUC 0.760) and STS-EACTS (AUC 0.763) were more powerful for predicting mortality and STS-EACTS for predicting prolonged LOS (AUC 0.733) and the need for high respiratory support (AUC 0.742).
    CONCLUSIONS: STS-EACTS seems to stratify better risk of mortality, prolonged LOS, and need for respiratory support after surgery.
    Keywords:  cardiac surgery; congenital heart disease; hospital mortality; intensive care; morbidity; risk adjustment
    DOI:  https://doi.org/10.1111/chd.12846
  7. Acta Paediatr. 2019 Sep 27.
    Verhagen AAEE.
      Paediatric palliative care (PPC) is an emerging specialty that aims at improving quality of life for patients and their families. The main goals of Paediatric palliative care (PPC) are to evaluate and minimize suffering in all domains (physical, psychosocial and spiritual), promote shared decision making and coordinate care. PPC is complex, and fundamentally different from adult palliative care in that it involves rare conditions, high-tech care, children in diverse developmental stages and complex proxy decision-making. One of the challenges of PPC today is to make sure patients receive continuity of care through the different stages of illness.
    DOI:  https://doi.org/10.1111/apa.15032